Motor Neuron's Disease

Question 1

Define Motor Neuron Disease:

Answer 1

This is a group of diseases characterised by rapid progressive motor neuron disorder of the voluntary muscle without any sensory symptoms.

Question 2

What is the basic pathology behind MND?

Answer 2

Selective loss of neurons in the motor cortex, cranial nerve nuclei and anterior horn cells.

Question 3

How can you distinguish between MND and myasthenia gravis?

Answer 3

MND NEVER affects the eyes

Question 4

What would indicate a poorer prognosis in MND?

Answer 4

Bulbar onset
Older age
Decreased FVC

Question 5

How is the gene associated with MND inherited?

Answer 5

Autosomal Dominant pattern

Question 6

What form of MND accounts for 87% of all familial MNDs?

Answer 6

Amyotrophic lateral sclerosis frontotemporal dementia locus

Question 7

What is the most common type of MND?

Answer 7

Amyotrophic lateral sclerosis (AML)

Question 8

Is AML mainly UMN or LMN disease

Answer 8

Mix of both

Usually one is dominant

Question 9

What diagnostic tool is used in the diagnosis of ALS?

Answer 9

El Escorial

Lower and Upper motor neuron signs in three regions is a definite diagnosis

Question 10

What clinical sign is commonly seen in ALS?

Answer 10

Split hand sign

Question 11

What is the average prognosis for ALS?

Answer 11

3-5 years

Question 12

What medication can be given in MND to prolong life?

Answer 12

Antiglutamatergic drug –> riluzole

Question 13

What % of patients with MND will suffer from frontotemporal dementia?

Answer 13

25%

Question 14

What is a pathological hallmark of ALS?

Answer 14

Inclusion bodies

Question 15

What is the classic onset of symptoms in ALS?

Answer 15

Focal onset in limbs then continuous spread
unilateral first
e.g. one leg then the trunk then the arm etc

Question 16

List the symptoms of an UMN lesion?

Answer 16

NO muscle atrophy
Spastic Gait 
Hyperreflexia
Present Babinski
Clonus 
Bradykinesia

Question 17

List some of the clinical features of a lower motor neuron lesion

Answer 17

Absent reflexes
Fasciculations
Muscle atrophy
Muscle weakness

Question 18

What form of MND presents with an Upper motor neuron lesion only?

Answer 18

Primary Lateral sclerosis

Question 19

What is the pathology behind primary lateral sclerosis?

Answer 19

Loss of Betz cells in the motor cortex

Question 20

What symptom is not seen in primary lateral sclerosis?

Answer 20

Cognitive decline

Question 21

What form of MND affects only the cranial nerves?

Answer 21

Progressive bulbar palsy

Question 22

What cranial nerves are affected in bulbar palsy?

Answer 22

CN IX - XII

Question 23

Is progressive bulbar palsy going to elicit upper or lower motor neuron signs?

Answer 23

Lower

Question 24

What medications can be given to MND patients as a management for drooling?

Answer 24

Amitriptyline

Propantheline

Question 25

What form of ventilation can be given to patients who suffer from MND?

Answer 25

Bi-phasic, non invasive, ventilation

Question 26

What form of MND only affects the anterior horn cells, leading to the symptoms being only LMN ?

Answer 26

Progressive muscular atrophy

Question 27

In progressive muscular atrophy how do the symptoms present?

Answer 27

Distal muscle groups are affected before proximal muscle groups.
Flail arm and flail leg
(tends to be restricted to one limb)
Better prognosis than ALS

Question 28

What is a common cause of death in patients with MND?

Answer 28

Respiratory failure due to aspiration pneumonia